Lung transplantation for lymphangioleiomyomatosis: the European experience

J Heart Lung Transplant. 2009 Jan;28(1):1-7. doi: 10.1016/j.healun.2008.09.014.


Background: Lung transplantation has been accepted widely as therapy for end-stage pulmonary lymphangioleiomyomatosis (LAM); however, single-center and national experience is limited due to the rarity of LAM.

Methods: We report the recent European experience of lung transplantation for LAM. A self-administered questionnaire was distributed to 30 European lung transplant centers to evaluate patients who underwent primary lung transplantation for LAM (1997 to 2007).

Results: Seventy percent of centers responded to the questionnaire. A total of 61 lung transplants were undertaken in women only, with mean age at transplant 41.3 years (SD 5.1). Centers performed a median of 2 (0 to 9) transplant operations. Severe pleural adhesions were the most common intra-operative complication. Early deaths (N = 6) were due to primary graft or multiple-organ failure or sepsis. Twelve recipients were diagnosed with bronchiolitis obliterans syndrome at a median of 20 months (range 10 to 86 months) post-transplant. LAM-related complications included renal angiomyolipoma and pneumothorax in the native lung. Recurrence of LAM occurred in 4 recipients. As of December 2007, actuarial Kaplan-Meier survival was 79% at 1 year and 73% at 3 years post-transplant.

Conclusions: Post-transplant outcome for pulmonary LAM in the recent era appears to have improved compared with the previous era. LAM-related complications remain common, but recurrence of LAM in the allograft is rare.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Cause of Death
  • Drug Therapy, Combination
  • Europe
  • Exercise Test
  • Female
  • Forced Expiratory Volume
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Intraoperative Complications / classification
  • Intraoperative Complications / epidemiology
  • Lung Diseases / epidemiology
  • Lung Neoplasms / surgery*
  • Lung Transplantation / mortality
  • Lung Transplantation / physiology*
  • Lymphangiosarcoma / surgery*
  • Respiratory Function Tests
  • Surveys and Questionnaires
  • Survival Analysis
  • Tissue Adhesions / epidemiology
  • Treatment Outcome


  • Immunosuppressive Agents